Rabbit Polyclonal antibody to Factor IThis gene encodes a serine proteinase that is essential for regulating the complement cascade. The encoded preproprotein is cleaved to produce both heavy and light chains, which are linked by disulfide bonds to form a heterodimeric glycoprotein. This heterodimer can cleave and inactivate the complement components C4b and C3b, and it prevents the assembly of the C3 and C5 convertase enzymes. Defects in this gene cause complement factor I deficiency, an autosomal recessive disease associated with a susceptibility to pyogenic infections. Mutations in this gene have been associated with a predisposition to atypical hemolytic uraemic syndrome, a disease characterized by acute renal failure, microangiopathic hemolytic anemia and thrombocytopenia. Primary glomerulonephritis with immmune deposits is another condition associated with mutation of this gene. [provided by RefSeq, Jul 2008]
Immunogen
KLH-conjugated synthetic peptide encompassing a sequence within the center region of human Factor I. The exact sequence is proprietary.
Key Feature
Clonality
Polyclonal
Isotype
IgG
Host Species
Rabbit
Tested Applications
WB
WB: 1:500 - 1:1000:
Species Reactivity
HumanMouseRat
Concentration
1mg/ml
Purification
Affinity purified
Target Information
Gene Synonyms
ARMD13 IF FI complement factor I C3b-INA C3BINA CFI KAF AHUS3
Target
Factor I
Tissue Specificity
Recognizes endogenous levels of Factor I protein.
Application
Application
Western blot analysis of Factor I expression in HEK293T (A); Raw264.7 (B); PC12 (C) whole cell lysates.
Application Notes
WB: 1:500 - 1:1000:
Additional Information
Form
Liquid
Storage Instructions
Shipped at 4 C. Upon delivery aliquot and store at -20 C for one year. Avoid freeze/thaw cycles.
Storage Buffer
Liquid in 0.42% Potassium phosphate, 0.87% Sodium chloride, pH 7.3, 30% glycerol, and 0.01% sodium azide.